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Goodpasture Syndrome !g2d-+pas-churz-!sin-+drOm

(Goodpasture Disease)

by Marvin I. Schwarz, MD

Goodpasture syndrome is a particular, uncommon autoimmune disorder in which bleeding into the lungs and progressive kidney failure occur.

  • People usually have difficulty breathing and cough up blood.

  • Laboratory tests on samples of blood and urine and a chest x-ray are needed to make the diagnosis.

  • Corticosteroids, cyclophosphamide (a chemotherapy drug), and plasma exchange are used to try to prevent permanent lung and kidney damage.

Goodpasture syndrome is a particular autoimmune disorder that typically causes kidney damage and diffuse alveolar hemorrhage (the pulmonary-renal syndrome). Occasionally Goodpasture syndrome affects only the kidneys or the lungs, but not both.

Goodpasture syndrome usually affects young men. Some people appear to be genetically susceptible. In these people, substances in the environment, such as tobacco smoke and some solvents, or a viral upper respiratory infection can cause them to produce proteins (antibodies) that react and try to destroy certain parts of their own bodies. These antibodies usually damage the tiny air sacs (alveoli) and the capillaries of the lungs and the filtering apparatus of the kidneys. The antibodies trigger inflammation that interferes with lung and kidney function.

Symptoms

A person with Goodpasture syndrome typically develops shortness of breath and coughs up blood. People may develop fever, lose weight without planning to, or develop anemia due to blood loss, resulting in fatigue.

Symptoms can quickly become severe. Occasionally symptoms are severe enough to cause the lungs to fail and people to have severe difficulty breathing, gasping, and bluish discoloration of the skin (cyanosis). When the lungs fail, the tissues of the body do not receive enough oxygen, and the person may die. Large amounts of blood can be lost. At the same time, the kidneys can rapidly fail. There may be small amounts of blood in the urine.

Diagnosis

Chest x-rays show abnormal white patches (due to lung bleeding) in both lungs. If the presence of lung bleeding is not clear based on symptoms and x-ray results (for example, if the person has not coughed up blood), doctors may need to insert a flexible viewing tube into the lungs (bronchoscopy) and wash out the lungs with fluid (bronchoalveolar lavage). Urine testing reveals blood and protein. Blood testing often shows anemia. Laboratory tests reveal the characteristic antibodies in the blood. Doctors usually remove a small piece of kidney tissue (biopsy) for analysis. The specimen shows microscopic deposits of antibodies in a specific pattern.

Treatment

Goodpasture syndrome may very rapidly lead to severe loss of lung function, a complete loss of kidney function, and death. High doses of corticosteroids and cyclophosphamide are given intravenously to suppress the activity of the immune system, and the person undergoes plasma exchange—a procedure in which the unwanted antibodies are removed from the blood (see Controlling Diseases by Purifying the Blood). The early use of this combination of treatments may help preserve lung and kidney function. Once damage occurs to the kidneys, it is usually permanent, and people may need dialysis or kidney transplantation.

Many people may need supportive care until the disease runs its course. For example, people may be given oxygen or may need help to breathe (using a mechanical ventilator) for a time. Blood or blood product transfusions may also be needed. If the kidneys fail, kidney dialysis or a kidney transplant may be required.

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  • CYTOXAN (LYOPHILIZED)